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Diseases

Genetic and Rare Diseases Information Center (GARD)

Exstrophy-epispadias complex


Other Names for this Disease
  • BEEC
  • Bladder exstrophy-epispadias-cloacal extrophy complex
  • EEC
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Overview

Exstrophy-epispadias complex (EEC) comprises a spectrum of congenital abnormalities that includes epispadias, classical bladder exstrophy and exstrophy of the cloaca and several variants.[1][2] EEC is characterized by a visible defect of the lower abdominal wall and other problems. The defect occurs due to a rupture of a fetal tissue known as the cloacal membrane during the first trimester of pregnancy. This results in the abnormal development of the abdominal wall of the fetus. The exact timing of the rupture determines whether the child is born with isolated epispadias, classic bladder exstrophy or cloacal exstrophy. Therefore, depending on severity, EEC may involve the urinary system, musculoskeletal system, pelvis, pelvic floor, abdominal wall, genitalia, and sometimes the spine and anus. There is no known cause for this condition.[1][3] Treatment may involve several surgeries to repair the abdominal wall and any associated malformation.[2][4]

The University of Michigan has a webpage about the development of the embryo and its parts, including the formation of the cloaca.
Last updated: 3/9/2016

References

  1. Cloacal exstrophy. Urology Care Foundation. 2016; http://www.urologyhealth.org/urologic-conditions/cloacal-exstrophy.
  2. Cloacal exstrophy. Orphanet. March, 2010. Available at: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=93929. Orphanet. March, 2010; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=EN&Expert=93929.
  3. Bladder Exstrophy-Epispadias-Cloacal Exstrophy Complex. NORD. 2007; http://rarediseases.org/rare-diseases/bladder-exstrophy-epispadias-cloacal-exstrophy-complex/.
  4. Yerkes EB. Exstrophy and Epispadias. Medscape Reference. http://emedicine.medscape.com/article/1014971-overview.
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Basic Information

In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
Other Names for this Disease
  • BEEC
  • Bladder exstrophy-epispadias-cloacal extrophy complex
  • EEC
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.